Challenges faced in the management of complicated Boerhaave syndrome: a tertiary care center experience

Spontaneous esophageal perforation is rare and is associated with high morbidity and mortality. A spectrum of various surgical modalities ranging from primary surgical repair to esophagectomy is available for its management. The optimal management of patients presenting late in a hemodynamically stable condition is not clearly defined in the literature. A retrospective review of all patients with Boerhaave syndrome managed by a single surgical team in a tertiary care center between 2008 and 2019 was performed (n = 16). Eleven patients were initially managed in the medical intensive care unit (MICU) as non-esophageal cause and 5 patients were referred after failed management (conservative/endoscopic). Demographics, clinical presentation, characteristics of perforation, initial diagnosis, and treatment were analyzed. All patients were males with a mean age of 42.2 years. A history of ethanol use was present in 6 patients. The median delay in diagnosis and referral was 16 days (range: 11-40 days). The common presenting symptoms were chest pain (n=11), dyspnoea (n=10), vomiting (n=4) and cough (n=2). The perforation was directed into right, left, and bilateral pleural cavities in 6, 8, and 2 patients respectively. The location of perforation was distal esophagus except for one patient. One patient was successfully treated with conservative management. The remaining patients underwent esophagectomy as a definitive surgical procedure. There was no significant postoperative morbidity and mortality. Esophagectomy can be done as a one-stage definitive procedure for patients with Boerhaave syndrome who present late in a hemodynamically stable condition with acceptable morbidity and good long term outcome.


Introduction
Spontaneous esophageal perforation (Boerhaave syndrome) is rare and is associated with high mortality [1]. Prompt recognition of this condition and early surgical management is associated with a good outcome. The first successful surgical management of Boerhaave syndrome was reported by N.R. Barrett in 1947 [2]. Since then, many treatment options (conservative, endoscopic, and surgical) have emerged. A spectrum of modalities ranging from primary surgical repair to more aggressive esophagectomy is available for the surgical management of esophageal perforation. However, the optimal management for Boerhaave syndrome remains controversial. In this study, we discuss the management of 16 cases of spontaneous esophageal perforation who were referred late in a stable condition and the role of esophagectomy in them.

Methods
A retrospective review of all patients with spontaneous esophageal perforation managed by a single surgical team between 2008 and 2019 was carried out. Patients with all other causes of esophageal perforation were excluded from the study. The following data were collected: demographic details of the patients, initial diagnosis and management in the ICU as non-esophageal thoracic cause, management details of the patients treated outside as Boerhaave syndrome, delay in diagnosis and referral, patterns of presentation, characteristics of the perforation, treatment offered, surgical approach for esophagectomy, morbidity and outcomes.

Results
A total of 16 patients were treated for Boerhaave syndrome. Eleven patients were initially managed in Medical ICU as non-esophageal thoracic cause and then referred to us after clinical suspicion of Boerhaave syndrome. Five patients were referred to us after failed surgical/endoscopic management for Boerhaave syndrome ( Figure 1).
All patients were males. The median delay in diagnosis was 16 days (Range: 11-40 days). The mean age was 42.4 (22-81 years). A history of alcohol use before the onset of symptoms was present in 6 (37.5%) patients ( Table 1). The initial presenting symptoms were chest pain (11), dyspnoea (10), vomiting (4), and cough (2). All patients (n=16) were hemodynamically stable. Out of 11 patients admitted in MICU, 6 patients were managed as pyothorax, 3 patients as pleural effusion, and 2 patients as unstable angina. The location of esophageal perforation was lower one-third in all patients except for one patient who developed tracheoesophageal fistula after endoscopic management of lower esophageal perforation. The perforation was directed into the right, left, and both pleural cavities in 6, 8, and 2 patients respectively. Out of 16 patients with Boerhaave syndrome, one patient was successfully treated with conservative management.

Discussion
In 1724, Hermann Boerhaave, a Dutch physicist, first described spontaneous rupture of the esophagus, which typically occurs after forceful emesis [3]. Spontaneous perforation of the esophagus also reported rates of mortality are between 16% and 24% [4], but it increases by up to 50% when treatment is initiated after 24 hours [5].
The most important survival predictor is the early onset of treatment.
Various treatment modalities available for esophageal perforation include conservative, endoscopic, and surgical methods. Surgical methods can either be primary surgical repair, diversion/exclusion procedures, and esophagectomy.
Endoscopic methods eliminate the morbidity and mortality associated with thoracotomy or laparotomy in a septic patient. Various endoscopic modalities for the management of perforation include hemoclip, OVESCO clip, endostitch, and SEMS placement. Stent migration is of specific concern in patients with Boerhaave syndrome as the stent has to cross the gastroesophageal junction to seal the perforation effectively. by a transthoracic approach in 9 patients [11]. Many studies [10][11][12][13][14][15][16] have proved esophagectomy as a better option for delayed esophageal perforation and are summarised in Table 3.

Conclusion
Our series demonstrates the effectiveness of esophagectomy as a one-stage procedure for the management of Boerhaave syndrome who present late and for those patients who present after failed endoscopic therapy. All our patients were hemodynamically stable and presented more than 10 days after perforation. In patients who survive the acute insult, esophagectomy as a one-stage surgical procedure is a better alternative in terms of morbidity and mortality.

What is known about this topic
• Early diagnosis and treatment is crucial for Boerhaave syndrome; • Patients who present within 24 hours of perforation have the best survival; • The management of patients who are diagnosed late (conservative/endoscopic/surgical) is unclear.

What this study adds
• In Boerhaave syndrome, if the patients survive the acute insult, surgical esophagectomy is a better treatment option than conservative/endoscopic management; • Transhiatal esophagectomy can be done as a one-stage definitive procedure for patients who present late in a hemodynamically stable condition with acceptable morbidity and good long term outcome.

Competing interests
The authors declare no competing interests.